β2-Adrenergic receptor agonists activate CFTR in intestinal organoids and subjects with cystic fibrosis

We hypothesized that people with cystic fibrosis (CF) who express CFTR (cystic fibrosis transmembrane conductance regulator) gene mutations associated with residual function may benefit from G-protein coupled receptor (GPCR)-targeting drugs that can activate and enhance CFTR function.We used intesti...

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Veröffentlicht in:	The European respiratory journal 2016-09, Vol.48 (3), p.768-779
Hauptverfasser:	Vijftigschild, Lodewijk A W, Berkers, Gitte, Dekkers, Johanna F, Zomer-van Ommen, Domenique D, Matthes, Elizabeth, Kruisselbrink, Evelien, Vonk, Annelotte, Hensen, Chantal E, Heida-Michel, Sabine, Geerdink, Margot, Janssens, Hettie M, van de Graaf, Eduard A, Bronsveld, Inez, de Winter-de Groot, Karin M, Majoor, Christof J, Heijerman, Harry G M, de Jonge, Hugo R, Hanrahan, John W, van der Ent, Cornelis K, Beekman, Jeffrey M
Format:	Artikel
Sprache:	eng
Schlagworte:	Administration, Oral Adrenergic beta-2 Receptor Agonists - pharmacology Albuterol - administration & dosage Biological Assay Bronchi - pathology Cell Line Cells, Cultured Chlorides - chemistry Cystic Fibrosis - drug therapy Cystic Fibrosis - genetics Cystic Fibrosis - metabolism Cystic Fibrosis Transmembrane Conductance Regulator - genetics Cystic Fibrosis Transmembrane Conductance Regulator - metabolism Drug Evaluation, Preclinical Epithelial Cells - metabolism Epithelium - metabolism Humans Mutation Nasal Mucosa - drug effects Nasal Mucosa - metabolism Organoids Pilot Projects Respiratory System - metabolism Signal Transduction
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